Braden’s Uphill Journey

Catching My Breath

Me, with my son, the weekend before I was hospitalized.

It started with stairs. Several months after the birth of my son, I carried a laundry basket up from our basement.  When I reached the nursery I was gasping for breath.  I sat down. Then I had to lie down—sides heaving, head swimming, heart pounding. Eventually my breathing slowed and I sat up, bewildered. I’m out of shape, I thought. I’m working too hard. I never get any sleep. Still, I called my doctor’s office.

The emergency care doctor was solicitous and kind. He gave me an EKG. He asked questions. Everything seems normal, he said. He talked about panic attacks. Babies are hard, he told me. I know, I said. He said to not feel silly for calling and he meant it … but I did.

Months passed. My son grew bigger and harder to carry. Every few weeks—then every week, then every few days—I would have another spell. I watched my boy draw deep, exaggerated breaths while we played together. He was imitating me. I called the doctor’s office again.

Bloodwork, EKG, everything seemed fine. I felt silly again. We should ultrasound your heart, my physician said. Maybe, I said, if these spells don’t stop.

They didn’t. I was slowing down—asking my husband to carry things, letting my son climb the stairs while I trudged up behind him. And then, while playing with my boy very early in the morning in October 2010, I fainted.

This time my doctor didn’t give me a choice about the ECHO. We have to find out what is going on, she said. I went in for the test. My cell phone started ringing before I finished the drive home. The right ventricle of your heart is significantly dilated, my doctor told me. The cardiologist needs to see you tomorrow. 

Braden, she said. I don’t want to scare you. But this is significant. We have to find out what is going on.

With my toddler climbing all over his office, the cardiologist told us I needed to check into Maine Medical Center immediately. We were stunned. It was our wedding anniversary! We called our families, who live out of state, on the drive to the hospital.  I told my mother, Mama, I’m so scared. When I said goodbye to my husband and son I wondered if I would ever see them again. I was covered in electrodes and wires and IV locks held down with tape. I crawled into my hospital bed and started to cry.

They starting running tests overnight. So many tests. I ran on treadmills, I breathed into tubes.  I spent two hours in a closed MRI. Images of my lungs were pressed like fingerprints on black screens in dim rooms. I watched saline bubbles foam like popped champagne through my swollen heart. My cardiologist sat at the foot of my bed and explained that the tests showed I didn’t have a hole in my heart, or blood clots in my lungs, or anything they had expected to find.

I have a hunch, he said. I think we need to explore pulmonary hypertension. When I told the nurses at shift change that night, they said they would pray he was wrong. Why? I wondered. So I Googled it.

The first few Google hits landed more like punches. Chronic high blood pressure in the lungs that puts untenable pressure on the heart, leading to right heart failure. No cause and no cure. Rare. Progressive. Fatal. Two years. Maybe five years. Lung transplants. Heart and lung transplants. Symptoms I recognized. Treatments that seemed they would invade my life but not save my life. I closed my laptop with shaking hands.

My son. My gorgeous, mischievous, sweet and sturdy son. Until my hospitalization we had never spent a night apart. Death would lock me alone in a dark room where I would hear him cry for me, but I would not be able to reach him. I sobbed. Margie, my night nurse, brought me a sleeping pill and held my hand. Those websites know nothing about you, she whispered.

I was given a right heart catheterization the next afternoon to confirm the diagnosis. Normal pulmonary artery pressure is 15 to 20 psi. The doctor performing the procedure measured mine at over 80. I’m sorry, he said, so sincerely, as I lay on the table with a probe still searching out the dark truths about my heart. You have pulmonary hypertension, and you can’t go home just yet. 

That same weekend, before we knew anything was truly wrong.

I Know Why You’re Here

My father flew across the country looking for any way to help. The news began to spread from family to friends to clients, but I didn’t want to see anyone until I could stop crying. Everyone wanted me to stop crying. The cardiologist let me wear my own clothes, take showers, take off the electrodes. My doctors came to see me, called my specialists for updates, told me funny stories about their kids. My wonderful husband brought treats from my favorite bakery on every visit. Together we walked our son around and around the ward, showing him the aquarium in the waiting room, pointing out the emergency helicopter landing on the roof outside my window.  

The nurses. Oh, the nurses. They were my best friends, my big sisters, my angels. 

Margie came running down the hallway in the early evening. Braden, she said, so excited. There’s somebody here you have to meet. Jeannette Morrill is here.

Who, I asked, is Jeannette Morrill?

Jeannette Morrill is one of the longest-lived survivors of pulmonary hypertension in the nation. Diagnosed at age 23 over 35 years ago, she was told she had two years to live. But against all odds and every expectation, she is still here. She travels from her home in northern Maine all over the country to advocate for PH awareness and a cure, and she has just written a book about her experiences called Living with Pulmonary Hypertension: 34 Years and Counting. She was having a painful flare up of rheumatoid arthritis, but she told Margie of course she would talk to me.

I have always been a visual learner. Until I met Jeannette, so strong and so warm and so alive, I could not see a future for myself with this disease. After I met her, that future was all I saw. I sat by her bed and she grasped my hand. Tell me, she said. And I did. The words just tumbled out until I was breathless all over again … but from excitement. From hope. She told me about her journey, a journey that began before there were treatments and support groups and conferences, when there was only sympathy.

She told me how she survived because she was a true responder—a very small percentage of PH patients who react to calcium channel blockers, basic blood pressure medications, years ago when that was all there was to take. True responders often see their symptoms reversed and their survival improve dramatically by taking just a few pills a day. These drugs had allowed Jeannette to adopt and raise two boys with her husband, David, and to adopt pulmonary hypertension as her cause. Eventually their efficacy waned and she moved on to more intense treatments, but she always persevered. Jeannette has been living a rich life with PH for as long as I’ve been alive.

“I don’t know why I’m still here,” she told me. I do, I thought, selfishly, but so gratefully. I do. I needed you to be here.  

I met my new pulmonary hypertension specialist, Dr. Wirth, the next day. He said we needed more tests to determine if I had primary PH or secondary PH (brought on by another so far undetected condition, like lupus, or sclerodoma). We needed to know if my PH had a cause, or if it was one of the truly rare cases called idiopathic, that strike out of nowhere for no known reason. He ordered another right heart catheterization to garner better measurements and to test my response to CCBs. I felt he was marshaling facts like soldiers, that we were laying in provisions for a good fight. He was very soft-spoken and he radiated calm. When I talked to him, I felt safe.

Buoyed up by knowing Jeannette was just down the hallway, that my husband was in my room waiting for me and that everyone I loved was praying I would be well, I almost refused the Valium before my second right heart cath. I had been told it was probably the last test I needed before I could go home and begin my treatment as an outpatient. That is what I focused on, lying on the gurney and then on the table, swaddled in heated blankets, pinned by the radiation shields. Home.

This cath was more complex than the first. I knew Dr. Wirth was watching from the observation deck, and that Dr. Ryan, the cardiologist, would be using two probes and taking more detailed measurements. I knew when the measurements were complete they would ask me to breathe nitric oxide through a mask for five minutes—a vasodilator challenge. If the blood vessels in my lungs opened and the pressures in my pulmonary artery decreased, I would be a true responder and able to take CCBs.

I had no expectations. I had only one thought: after this, you can go home.

Into the Night of My Very Own Room

This isn’t like going to the dentist, the anesthesiologist explained. You won’t smell anything or taste anything or start feeling funny. Just take deep breaths into this mask for five minutes. Okay?

Okay.

I closed my eyes and drew that first deep breath. Then another. And another. It was so calming to just breathe. I could feel each breath coming from a deep place, and then an even deeper place. Another breath. And another. It was so simple to breathe. I hadn’t realized. I had been doing it wrong for so long.

I heard Dr. Ryan’s voice. Did it really go down that much? He asked. I kept breathing. Each breath drawn up, fresh and cool, from a new well. Again. And again.

Wow, someone said. Wow. I kept breathing, but I was doing something else, now—I was hoping.

The anesthesiologist pulled the mask away from my face and I realized that it wasn’t just me—the entire energy in the room had changed. One of the women on the team beamed at me. Good job! she said, her eyes wet with tears. You did such a good job!

Dr. Wirth came down from the observation deck. He was smiling, too. Did it work? I asked him. Did I do a good job?

 Your pressures went down to a normal range, he said. Yes, Braden, it worked.

The easy breathing was leaving me, the effects of the nitric were fading. But something bright was blooming in my chest. People were squeezing my shoulders, stroking my hair. I thought—you are all in this moment I will never, ever forget. Some part of me will always be in this room, on this table and under these lights, measuring the life I thought I’d lost against the life you just returned to me.

Thank you, I kept saying. Thank you.

They wheeled me into an adjoining room to begin the process of applying pressure to the cath site and start my four hours of post-procedure bed rest. When we rolled through the swinging doors I saw one of the members of my initial cath team—a young woman who had asked me, after my diagnosis had first been confirmed, if I wanted any extra Valium. There was something so sweet and pragmatic and right about that question at just that moment that I had felt better instantly. I smiled at her. I was so glad to see her. She reached out to me as they wheeled me past.

You are going to live a long and happy life, she told me. And that’s when I started to cry.

The summer after my diagnosis, after testing showed my pressures back in normal range.

The first night I was home, after my son’s bath, the three of us sat cuddled together in a pool of lamplight in the nursery and read his bedtime story. He chose, as he almost always does, Where The Wild Things Are. My son turned the pages, my husband roared whenever a Wild Thing appeared and I read the book. But my voice kept getting caught at the end, on the description of Max’s return journey from where the Wild Things are, when he “sailed back over a year, and in and out of weeks, and through a day, and into the night of his very own room.”

Reading the book as a child it seemed ridiculous that anyone could have such an extraordinary experience and return to find their normal, everyday life at the exact place they left it. But I know better, now. We kissed our son good night and went downstairs to the kitchen, where my supper was waiting for me, and it was still hot.